What Is Hypogammaglobulinemia?

Hypogammaglobulinemia is a condition that affects your body’s ability to make immunoglobulins (Ig), or antibodies. Antibodies prompt your body’s immune system to protect you from viruses, bacteria, and other sources of illness. With fewer antibodies available, people with hypogammaglobulinemia have a much higher risk of infection and disease.

Hypogammaglobulinemia is often inherited due to genetic abnormalities. In some cases, people develop hypogammaglobulinemia due to external causes, such as chemotherapy, certain comorbid disorders (those that occur along with hypogammaglobulinemia), or the use of immunosuppressants. Some patients also acquire new mutations (for poorly understood reasons) that lead to the development of this condition. Treatment options include antibiotics and/or immunoglobulin replacement therapy to increase antibody levels and improve immune response.

Learn more about hypogammaglobulinemia, including symptoms, causes, diagnosis, and treatment options.

Hypogammaglobulinemia Symptoms

Hypogammaglobulinemia is an immunodeficiency disorder, which means it affects the body’s ability to prevent infection.  Some people with hypogammaglobulinemia begin to experience symptoms during childhood. However, your symptoms may not show up until adulthood, typically during your 20s and 30s.

People with hypogammaglobulinemia have both an increased risk of infection and increased risk of the number of infections they get, especially:

• Lung infections
• Sinus infections
• Gastrointestinal infections
• Bronchitis (upper airway infection)
• Ear infections
• Tonsillitis (tonsil infection)
• Conjunctivitis (pink eye)

People with untreated hypogammaglobulinemia may also experience a number of medical complications. Some complications may be serious, including:

• Frequent, severe diarrhea
• Allergies
• Asthma
• Autoimmune disorders, which cause your body's immune system to attack healthy cells
• Hypothyroidism (a type of autoimmune disorder)
• Pneumonia
• Bronchiectasis (chronic bronchitis and excessive mucus buildup)
• Malnutrition and difficulty absorbing nutrients from food
• Problems with liver function
• Enlarged spleen
• Swollen, painful joints
• Swollen, tender lymph nodes
• Blood disorders, such as thrombocytopenia (low platelet count)
• A higher risk of certain cancers

Causes

Primary or inherited hypogammaglobulinemia is caused by genetic factors, such as genetic mutations or chromosomal abnormalities. Usually, this means that your body struggles with producing and maintaining healthy B cells and/or T cells (types of white blood cells).

Secondary hypogammaglobulinemia can be caused by anything that impairs your body’s immune response or its ability to make antibodies, such as:

• Immunosuppressive drugs and treatments, such as corticosteroids and chemotherapy
• Exposure to radiation
• Organ transplant
• Cancer
• Protein-losing enteropathy (PLE), which causes protein loss from the gastrointestinal tract
• Nephrotic syndrome, a kidney disorder that leads to protein loss
  

Diagnosis

Your healthcare provider can make a diagnosis of hypogammaglobulinemia using a physical examination, an assessment of your medical history (such as a history of recurring infections), and a series of lab tests. Blood tests may reveal low levels of certain antibodies, such as immunoglobulin G (IgG). You may also develop lower than expected levels of antibodies than expected after vaccination.

Treatment

If you have secondary hypogammaglobulinemia, treating the underlying cause will help to reduce your symptoms. For example, you may experience fewer symptoms once you stop taking immunosuppressants or after your chemotherapy treatments have ended.

Treatment for primary hypogammaglobulinemia typically involves antibiotics and/or immunoglobulin therapy. 

Antibiotics can help to relieve the symptoms of frequent infections. Your healthcare provider may also prescribe prophylactic antibiotics, which you can take on a consistent basis to prevent (rather than treat) infection.

Immunoglobulin replacement therapy involves regular infusions that can increase the level of antibodies in your body. This can help to improve your quality of life and prevent infection, as well as related medical complications. 

Immunoglobulin infusions can be delivered intravenously (through an IV) or subcutaneously (via injection). Intravenous Ig treatment (IVIg) is usually given at the hospital, often two to four times per week for two to four hours at a time. IVIG is also given in infusion clinics in outpatient centers set up to deliver this medication. Usually, IVIG is monthly. Subcutaneous Ig treatment (SCIg) is typically delivered at home and may require more frequent doses.

Summary

Hypogammaglobulinemia is a medical condition that leads to low levels of immunoglobulins, or antibodies, in the blood. Low immunoglobulin levels reduce your body’s ability to fight off infection and prevent disease. 

People with hypogammaglobulinemia frequently experience recurring respiratory and gastrointestinal infections. Lung infections, sinus infections, and upper airway infections are especially common. Hypogammaglobulinemia is diagnosed with blood tests that measure immunoglobulin levels. Treatments usually involve antibiotics and immunoglobulin infusions to increase antibody levels over time.

A Word From Verywell

If you have frequent infections, you may have an immunodeficiency disorder such as hypogammaglobulinemia. If left untreated, you could experience severe medical complications. It’s important to talk to your healthcare provider as soon as possible about your treatment options.